This presentation discusses the case of a 55-year-old male presenting with symptoms of anemia, fever, frequent urination, and multiple neck and axillary swellings. His history includes recent diagnoses of diabetes and hypertension. Examination found palpable lymphadenopathy. Investigations including lymph node biopsy led to a diagnosis of multicentric Castleman's disease, plasma cell variant. The presentation provides details on the patient's history, examination findings, test results, diagnosis, and discusses Castleman's disease including its epidemiology, etiology, classification, and management.

1. GRAND ROUND
PRESENTATION
Presenters:
AME ISMAIL
ROSE MINJA
ZAHRA KHAN

2. PATIENT PARTICULARS
• Name: M.S.K
• Age: 55 years
• Sex: Male
• Residence: Korogwe- Tanga
• Occupation: Civil and irrigation engineer
• DOA: 15/2/2016
• DOD:25/2/2016.
• Referral: Bombo Regional Hospital > MNH > ORCI

3. CHIEF COMPLAINTS
Awareness of heartbeats
Fever
Frequent urination and thirst
Multiple swelling on the neck
6/12

4. HPI
• Awareness of heartbeat
• Insidious onset
• No specific periodicity
• Associated with GBM and dizziness
• With a single episode of LOC (as reported by his wife)
• 2-3 hr, not acc.with convulsion, tongue bite ,stool or urine
incontinence
• Not postural related
• Neither associated with chest pain nor cough
• No hx of air hunger at night or DIB
• Not associated with bilateral lower limb swelling

5. HPI
Fever
• Gradually
• Low grade
• No specific periodicity,
• Hx of night sweat
• No Hx of yellowish discoloration of the eyes, or skin
• No history of headache
• No history of body rash
• No history of joint pain, or bone pain or recurrent sore throats
• Temporarily relieved by PCM

6. HPI
Frequent urination
• Gradual onset
• More during the night
• Associated with excessive thirst ,loss of appetite and loss of
weight (clothing)
• Not painful /blood stained
• No Hx of vomiting , diarrhea, constipation or blood in stool
• No hx of loss of vision, libido, erectile dysfunction ,numbness
or tingling sensation or joint pain

7. HPI
Multiple swellings on the neck and axilla
• Gradual onset
• Started on the Rt side of the neck, later appeared on the Lt side
of the neck and Lt axilla
• increasing and decreasing in size and number
• Mobile ,Wax and waning course
• Not warm , not painful, no skin changes, not ulcerating
• Not associated with hoarseness of voice or difficulty in
swallowing

8. Denied history of
• Pruritus
• Easily bruising, nose or gum bleeding
• Joint, bone or back pain
• Long term medication use eg: phenytoin, radiation or
chemotherapy
• BT prior to sickness

9. History of Presenting Illness
At Bombo Hospital Hospital, Sept 2015
• Admitted for 10days
• Diagnosed with Dm and HTN
• Received 2unit of blood, losartan, Inj. insulin (during acute
phase) and Lasix
• Mild improvement
A month later -Regency Hospital,
• Admitted for 23days
• OGD and colonoscopy, hypothyroidism and was treated for
HeligoKit, caps ferotone, metfomin and L-thyroxine
• Received 3units of blood

10. Bombo Hospital Hospital, february 2016
• GBM ,dizziness and palpitation
• HB 2g/dl, RBG 2mmol/l
• Received 2units of blood
• Referred to MNH with the suspicious of leukemia
Progress in the ward
• Received 2unit of blood, lymph node biopsy investigated
discharged through OPD

11. Past Medical History
• 1st admission Bombo Hosp.
• 2nd Regency MH
• 3rd at MNH
• Recurrent BT 7times
• No known drug or food allergies
• New Dx HTN, DM and hypothyroidism
• HIV test done -ve

12. DIETARY HX
• Mainly 3 meals per day
– B/fast:
o1-2 cup of black tea with toast/andazi/chapati
• Lunch:
o One plate Ugali/Rice/Banana, with beans and vegetables,
meat, fish
• Dinner:
o Same as lunch
o Fruits: frequently: seasonal [orange and ripe bananas]
• Conclusion: Adequate but reduced due to the current illness

13. FSHx
• Married with three children
• University graduate
• Working as a civil and irrigation engineer
• All his children are well
• He has two sisters and three brothers, all are well
• No history of the same illness in the family
• No hx of DM, HT in the family
• No Hx of smoking, alcohol use or drug abuse

14. SUMMARY
• M.S.K, 55yrs from Tanga, presenting with 6/12 with
symptoms of anemia, B symptoms, multiple neck and axillary
swellings which were waxing and waning, and hx of multiple
BT(7times)
• Recently diagnosed with DM and HTN on medication

15. General Examination 19/02/2016
• Oriented PPT
• Afebrile T 36.90C
• Pale +++
• Not jaundice
• Not dehydrated
• Not cyanotic
• Ø Oral ulcers/ thrush
• Palpable lymphnode
• No finger clubbing
• Ø Lower limb oedema

16. Lymph nodes exam
They are round, rubbery, largest measuring 4*3 cm, non tender, not
warm, not matted, mobile and the overlying skin is normal
LN Right side and
number LN
Left side and number
of LN
Cervical Posterior 1 (4*3) Posterior (1)
Anterior 2
Submandibular 1 -
Sumental 1(1cm)
axillary 1(1cm) -
Inguinal 2(1-2cm) 4(2-3cm)

17. Systemic Examination
CVS
o PR 98 bpm, regular, good volume ,no collapsing,
synchronous
o JVP not raised
o No precordial mass/deformity
o No precordial hyperactivity
o Apex beat 5ICMCL
o Normal S1 & S2 ,no added sound
o Conclusion : normal CVS examination

18. Systemic Examination
• RS:
o RR 16breath/min
o No therapeutic marks or scars
o Normal chest movement
o Normal chest expansion
o Trachea central located
o No areas of tenderness elicited
o No gynaecomastia

19. Systemic Examination
• RS
o Normal tactile vocal fremitus
o normal percussion note
o Normal vocal resonance
o Normal air entry and normal vesicular breath sounds
o Conclusion: Normal RS examination

20. Systemic Examination
P/A
o Not distended
o Therapeutic marks
o Moves with respiration
o No visible peristalsis
o No distended veins
o No mass on superficial palpation
o No tenderness

21. Systemic Examination
P/A
o kidneys not ballot able
o Splenomegaly 2cm below Left costal margin to the notch,
firm ,smooth, roundborder, moves with resp
o Liver not palpable, span 13cm
o Tympanic percussion note
o Normal bowel sound
Genitalia- normal testes and skin around it
o DRE normal sphincter tone, gloves stained with normal
color of stool

22. Systemic Examination
• CNS
o Fully conscious
o Oriented to TPP
o Normal speech
o Intact short and long-term memory
• Conclusion: Intact higher centers

23. Systemic Examination
• CNS
o Can smell normally
o Can see normally
o Can move eyes in all directions
o Pupils are normal in size and reacting to light normally
o Can clench teeth with normal sensation in the face
o Normal facial expression
o Normal hearing and balance
o Can swallow normally
o Can turn neck sideways against resistance
o No tongue deviation
• Conclusion: normal cranial nerves

24. Upper Limbs
• Motor System 
– Bulk: normal muscle
– Ø Fasciculation
– Tone: N
– Power 5/5 :all grps of muscles
– Reflexes: normal
• Coordination : normal
• Sensation : normal

25. Lower limb
• Motor system
– Bulk: normal muscle
– Ø Fasciculation
– Tone: Normal
– Power 5/5 :all groups of muscles
– Reflexes: Normal
• Coordination : normal
• Sensation : normal
• Conclusion : normal motor and sensory function

26. Summary
• M.S.K, 55yrs from Tanga, presenting with 6/12 with symptoms of
anemia, B symptoms, generalized lymphadenopathy which were
waxing and waning, and hx of multiple BT(7times )
• Recently diagnosed with DM and HTN on medication.
O/E
• Pale+++, painless palpable lymphadernopathy of variable size in
cervical, submandibular, sub mental, axilla, inguinal and
splenomegaly.

27. Provisional diagnosis

28. 1. Chronic Lymphocytic leukemia
Positive
• Commonest
• Male (2X) , age >50 years
• Insidious onset
• Peripheral lymphadenopathy + Splenomegaly
• Symptoms and signs of anemia
• Anorexia, night sweats and fever
Negative
• SX of thrombocytopenia

29. Non Hodgkin Lymphoma
Positive
• Age (median age 50 years)
• Constitutional symptoms (40%)
• Anemia (BM involvement occurs in 60%)
• Peripheral LN enlargement (2/3)
Negative
• Low grade fever
• Severe anemia
• Hepatomegaly (40% of indolent NHL)

30. Tb Lymphadenitis
Positive
• Constitutional symptoms
• Peripheral lymphadenitis
• Symptoms and signs of anemia
Negative
• Presence of severe anemia
• LN description ( not matted, warm and inguinal LN are rarely
involved)

31. Giant LN hyperplasia(Castleman disease)
Positive
• Age (mean age 50 -65years)
• Male (65 -75%)
• Insidious onset (MCD variant, HIV -VE)
• Wax and wane(MCD, plasma cell variant)
• Inflammatory symptoms (90%)
• Peripheral LN splenomegaly(80 – 92%)
• Anemia
Negative
• Respiratory Sx/ effusion
• Severe anemia

32. POEMS Syndrome
Positive
• Splenomegaly and lymph
node enlargement
• Hypothyroidism and
hyperglycemia (T2DM)
• Constitutional symptoms
• Anemia
• Male (2.5:1)
Negative
• Monoclonal plasma
proliferative disorders
• Polyneuropathy/pulmonary sx
• Skin changes
(hyperpigmentation/clubbing)
• Ocular sx and signs
• Severe anemia

33. 2.DM + Hypertension
• Diagnosed
• On treatment
• Symptoms of polyuria and polydipsia

34. Investigations

35. FBP
10/1/2016 23/1/2016 21/2/2016 9/4/2016
WBC 10.42 4.32 7.73 4.41 4-11K/ML
NEUTROPHIL ABs 6.58 1.39 3.54 2.32 2-6.9K/ML
LYMPHOCYTES Abs 3.42 3.46 4.10 4.19 0.6-3.4K/ML
MONOCYTE Abs 0.45 0.82 1.53 0.35 0-0.9K/ML
EOSINOPHIL Abs 0.07 0.07 0.09 0.05 0.0-0.7K/ML
RBC 0.86 2.23 0.92 1.80 4.69-6.07K/ML
HB 3.2 2.05 3.30 5.17 14.6-17.8G/dl
HCT 8.60 21.5 8.65 17.7 40.8-51.9%
MCV 88.6 96.3 93.6 98.1 80-100FL
MCH 28.1 31.6 28.3 28.7 27-31Pg
RDW 16.7 21.5 18.4 23.9 10-15%
PLATELETS 279 326 369 446 140-450K/ML

36. Biochemistry
Sodium 133
ALT 36 0-55u/l
Phosphorous 0.8 0.74-1.2mmol/l
Creatinine 67 60 – 120umol/L
Chloride 101 99 – 107mmol
Potassium 3.7 3.5 – 5mmol/L
Total protein 84 60-80g/l
Albumin 32 35-50g/l
BUN 4.8 3.2-7.4mmol/l
Calcium 2.18 2.1-2.5mmol/l
AST 32 5-35u/l

37. Bleeding indices

38. Lipid profile

39. Hormonal Assay (TFT)
TEST RESULT NORMAL RANGE
TSH 4.3 3 – 7.0ng/ml
T3 4.69 4 – 6.5 pg/ml
T4 13.3 10 – 23 pmol/L

40. Serology
• HIV 1 and 2 Antibody - negative
• HBV surface Antigen - negative
• HBV surface antibody- negative
• HBC surface antibody - negative
• PCR for HHV 8 = negative

41. ESR
• Erythrocyte sedimentation rate – 106mm/hour

42. Peripheral blood smear
• RBC - normocytic normochromic
• WBC – Adequate
• Platelet – adequate
• Reticulocyte count – 1.9%

43. Bone marrow aspiration and cytology
• Cellularity – normocellular particles and trails
• Erythropoiesis increased
• Sideroblasts not seen
• Iron stores – weak
• Myeloid : Erythroid 1:2
• Granulopoiesis – well presented
• Megakaryocytes – mild increased

44. BMAC…
• Free flying platelets – seen
• Plasma cells – seen in 1% of myelogram
• Lymphocytes – mild increased
• Monocytes – not increased
• Abnormal cells/blasts cells- Blasts consists of 1% of
myelogram
CONCLUSION: Features suggestive of Erythroid
hyperplasia with reduced iron stores

45. LN biopsy

46. LN biopsy

47. Lymph node biopsy
Section covered by thin capsule showed proliferation of follicles
with follicular and cells in onion skin appearance, hyalinized
interfollicular blood vessels, extensive proliferation of plasma
cells
CONCLUSION:
Multicentric Castleman disease, plasma cell variant

48. ECG

49. CXR

50. Abdominal USS

51. Urinalysis

52. Wish list
• IL – 6 level
• Vascular Endothelial Growth factor (VEGF)
level
• Protein electrophoresis

53. Final diagnosis
• Multicentric Castleman disease, plasma cell variant, HHV 8
negative with anemia
• Type 2 DM
• HTN

54. CASTLEMAN’S DISEASE
DISCUSSION AND MANAGEMENT

55. Castleman disease
• Castleman disease (CD) is a rare d’se of lymph nodes and related
tissues.
• Other names -Giant lymph node hyperplasia, angiofollicular lymph
node hyperplasia (AFH).
• It was first described by Dr. Benjamin Castleman in the 1950s.
• It is a heterogenous group of lymphoproliferative disorders that are
sometimes associated with HIV and HHV-8.
• CD is not cancerous but it may also be associated with malignancies
such as KS, NHL, HL and POEMS syndrome.
• The d’se is nonclonal.

56. Epidemiology
• CD is rare.
• No sexual predilection, affects all ages, rare in children.
• The plasma cell type has a greater prevalence among young males
and females.
• The mean age of pts with MCD is 50-65 years.
• Persons with HIV infection may be younger.
• Males account for 50%-65% of MCD cases.
• The incidence of MCD has increased with ART for the mx of HIV.
• On multivariate analysis,risk factors of MCD:* :CD4 count > 200/µL,
Increased age ,No previous ART exposure , Nonwhite ethnicity
* Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005 Apr. 129(1):3-17.
[Medline].

57. Etiology
• The exact cause is unknown.
• An increased production of IL-6 by lymph nodes
appears to have a role in the development of CD.*
• HHV-8 and release of IL-6 or related polypeptides
also appears to have a role.**
*Leger-Ravet MB, Peuchmaur M, Devergne O, Audouin J, Raphael M, Van Damme J, et al. Interleukin-6 gene expression in Castleman's disease. Blood. 1991
Dec 1. 78(11):2923-30. [Medline].
**Dupin N, Diss TL, Kellam P, Tulliez M, Du MQ, Sicard D, et al. HHV-8 is associated with a plasmablastic variant of Castleman disease that is linked to HHV-8-
positive plasmablastic lymphoma. Blood. 2000 Feb 15. 95(4):1406-12. [Medline].

58. Pathophysiology
• Nodal expansions that usually leave the structure of the
underlying lymph node at least partially intact. B cells and plasma
cells are polyclonal, and T cells show no evidence of an aberrant
immunophenotype.
• It can be grouped in numerous ways, as discussed below.
1. Localized versus multicentric Castleman disease
• Localized (unicentric) CD- More common type, affecting only a
single group of lymph nodes, usually in the chest or abdomen.

59. Pathophysiology
Multicentric Castleman disease (MCD) - affects more than one
group of lymph nodes.
• It can also affect organs that contain lymphoid tissue.
• This form is often associated with HIV and HHV-8 and results in
systemic symptoms: serious infections, fevers, weight loss,
fatigue, night sweats, and neuropathy.
• Anemia and hypergammaglobulinemia are common. In addition,
MCD may transform to lymphoma.

60. Pathophysiology
2. Microscopic subtypes
• The hyaline vascular type - most common type ≈ 90% .It is
localized and causes few symptoms. The prognosis is typically
good, but, in rare cases, it may be multicentric.
• The plasma cell type - symptomatic and multicentric but may be
localized. Histologically, there are sheets of mature plasma cells
within interfollicular tissues that surround normal to large
germinal centers. Dysregulation of IL-6 has been implicated in its
pathogenesis.[*, **]
*Yoshizaki K, Matsuda T, Nishimoto N, Kuritani T, Taeho L, Aozasa K, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease. Blood. 1989
Sep. 74(4):1360-7. [Medline].
**Leger-Ravet MB, Peuchmaur M, Devergne O, Audouin J, Raphael M, Van Damme J, et al. Interleukin-6 gene expression in Castleman's disease. Blood. 1991 Dec 1.
78(11):2923-30. [Medline].

61. Pathophysiology
• In the mixed subtype, there are areas of both hyaline vascular
and plasma cell types. This is a rare subtype .
• The plasmablastic type of CD is usually multicentric and
symptomatic. It has a less favorable prognosis.
3. Subtypes based on viral infections
• Infection with HIV, HHV-8, or KSHV plays a role in at least some
cases.
• MCD is more common in ppl with HIV infection. MCD is often
subclassified based on HIV/HHV-8 status.

62. Clinical features
• Unicentric CD is generally asymptomatic but may cause Localized
lymphadenopathy with resultant compressive sx, systemic sx.
• MCD- < 10% are asymptomatic, multiple lymphadenopathy
and/or the following systemic sx: Fever, night sweats, Weight
loss, loss of appetite , weakness , fatigue, SOB, cough, Nausea
and vomiting , neuropathy, Leg edema, Skin rashes,
Hemangiomata, Pemphigus, Kaposi sarcoma, splenomegally
• Other conditions associated with MCD - amyloidosis or POEMS
syndrome, autoimmune d’se, hemolytic anemia, ITP, and
acquired factor 8 deficiency.
• Some of these symptoms might remit and relapse over time.

63. Laboratory Studies
• CBC : For anemia (usually mild to moderate, occasionally, < 8
g/dL), thrombocytosis
• LFT: Hypoalbuminemia
• Serum protein electrophoresis: For a polyclonal
hypergammaglobulinemia
• ESR: Usually elevated
• Serologies for hepatitis B, HHV-8, and HIV with quantitative
assays, if positive
• IL-6, VEGF, LDH and CRP levels: High
• HHV-8 viral load study or immunohistochemistry for HHV-8 in the
lymph node

64. Investigations
• Bx of lymph node - histopathology, flow cytometry, cytogenetics, FISH
for lymphoma studies, and B-cell gene rearrangement studies.
• CXR- In MCD may show bilateral reticular or ground-glass opacities,
mediastinal widening, and/or bilateral pleural effusions.
• Chest CT scans- lymphadenopathy of multiple enlarged mediastinal
and hilar lymph nodes (1-3 cm in diameter). Lung parenchymal
findings including subpleural nodules, interlobular septal thickening,
peribronchovascular thickening, ground-glass opacities, and patchy
rounded areas of consolidation. Small to moderate bilateral pleural
effusions may also be present.
• CT scanning of the neck, abdomen, and pelvis.
• PET- used for staging

65. Treatment- Unicentric CD
• Surgery is usually curative. In pts whose lesions cannot be
completely resected, outcomes remain favorable. Partially
resected masses may remain stable and asymptomatic for many
yrs.
• Pts with unresectable diseases with compressive symptoms can
be treated as described for HIV-negative MCD.
• Systemic steroids can provide symptomatic relief but do not
predictably reduce tumor size.
• Radiation therapy - result in complete and partial remission rates
of 40% and 10%, respectively, but can cause radiation-induced
fibrosis that makes subsequent surgical intervention more
difficult.*
*Chronowski GM, Ha CS, Wilder RB, Cabanillas F, Manning J, Cox JD. Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy.
Cancer. 2001 Aug 1. 92(3):670-6. [Medline].

66. Treatment - MCD
• No standard therapy , and clinical practice varies.
• Therapy depends on HIV/HHV-8 status and then on the clinical
aggressiveness of the d’se. Treatment options include are:
IL-6-directed therapy
• Especially in HIV/HHV-8–negative pts with mild symptoms and no
organ failure, immunotherapy with monoclonal antibodies directed
at IL-6 (siltuximab) or the IL-6 receptor (tocilizumab) was reported to
yield a 2-yr overall survival rates and relapse-free rates of 94%-95%
and 79%-85%, respectively.[*, **]
• Anti–IL-6–directed treatment is continued until progression of d’se in
order to maintain the response and prevent an early relapse.
• If neither agent is available- anti-CD20 monoclonal antibody.
*Beck JT, Hsu SM, Wijdenes J, Bataille R, Klein B, Vesole D, et al. Brief report: alleviation of systemic manifestations of Castleman's disease by monoclonal anti-interleukin-6 antibody. N Engl J
Med. 1994 Mar 3. 330(9):602-5. [Medline].
**Kawabata H, Tomosugi N, Kanda J, Tanaka Y, Yoshizaki K, Uchiyama T. Anti-interleukin 6 receptor antibody tocilizumab reduces the level of serum hepcidin in patients with multicentric
Castleman's disease. Haematologica. 2007 Jun. 92(6):857-8. [Medline].

67. Treatment- MCD
Anti-CD20 monoclonal antibody therapy
• Rituximab with or without steroids and/or chemotherapy used
regardless of HIV status and yields a good response, especially when
used along with chemotherapy.
• Rituximab can worsen KS, so carefully considered in HIV-positive pts
with high viral load, low CD4 count, and active KS.
Cytotoxic chemotherapy
• Vinblastine and etoposide, both as single agents, yielding
symptomatic relief and a partial response in almost all pts.*
• However, sx recur when treatment is stopped, necessitating
intermittent maintenance therapy, often lifelong. Thus, combination
chemotherapy is preferred to monotherapy.
• Etoposide is often used with rituximab in HIV/HHV-8–positive pts
with organ dysfunction and aggressive d’se.
*Bower M, Powles T, Williams S, Davis TN, Atkins M, Montoto S, et al. Brief communication: rituximab in HIV-associated multicentric Castleman disease. Ann Intern Med. 2007 Dec 18.
147(12):836-9. [Medline].
**Bower M. How I treat HIV-associated multicentric Castleman disease. Blood. 2010 Nov 25. 116(22):4415-21. [Medline]

68. Treatment in HIV/HHV-8–positive
patients
• Combination of ganciclovir plus rituximab, with etoposide
added for symptomatic or aggressive d’se.*
• ART therapy is included with the above combination regimen
in pts with a low CD4 count, higher HIV load,active KS.
• Other therapies with limited efficacy data include the
following:
- Antiviral therapy (ganciclovir, cidofovir, interferon alpha)
- Bortezomib: activity in the plasma cell variant**
- Thalidomide plus rituximab: Induce some responses ***
*Uldrick TS, Polizzotto MN, Aleman K, O'Mahony D, Wyvill KM, Wang V, et al. High-dose zidovudine plus valganciclovir for Kaposi sarcoma herpesvirus-
associated multicentric Castleman disease: a pilot study of virus-activated cytotoxic therapy. Blood. 2011 Jun 30. 117(26):6977-86. [Medline].
**Sobas MA, Alonso Vence N, Diaz Arias J, Bendaña Lopez A, Fraga Rodriguez M, Bello Lopez JL. Efficacy of bortezomib in refractory form of multicentric
Castleman disease associated to poems syndrome (MCD-POEMS variant). Ann Hematol. 2010 Feb. 89(2):217-9. [Medline].
***Wang X, Ye S, Xiong C, Gao J, Xiao C, Xing X. Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric
mixed-type Castleman's disease. Jpn J Clin Oncol. 2011 Oct. 41(10):1221-4. [Medline)

69. Management
• Blood transfusion- 3 PRBC
• Prednisolone 60mg OD
• Paracetamol 1g PRN
• Chemotherapy

70. Monitoring and follow up
• Monitor RBG regularly – Last 4.9 mmol/L
• Watch out for side effects of Prednisolone
– Gastritis/PUD, Oral ulcers, Osteoporosis, Weight gain, Cataracts,
Easy bruising, DM
• D’se response is assessed with imaging and lab data after about 4
cycles of therapy. A second round may be administered upon a
partial response.
• Monitoring at periodic intervals (2-4 months) with a hx and PE and
serum biomarkers (IL-6, CRP, serum free light chain assay,
quantitative immunoglobulins).
• Generally, annual imaging can be discontinued after 5 yrs if the pt
remains d’se-free.

71. Prognosis and councelling
• It varies based on the type.
• The prognosis in unicentric is excellent.
• MCD - variable prognosis, from indolent d’se to an episodic
relapsing form to a rapidly progressive form leading to death
within weeks (HIV infection).
• A 2011 meta-analysis by Talat et al reported 3-year d’se-free
survival :*
Class I (unicentric, hyaline vascular, HIV-negative): 93%
Class II (plasma cell unicentric , mixed-pathology unicentric, or
multicentric hyaline vascular [all HIV-negative]): 79%
Class III (multicentric, plasma cell, HIV-negative): 46%
Class IV (HIV-positive [multicentric]): 28%
* Talat N, Schulte KM. Castleman's disease: systematic analysis of 416 patients from the literature. Oncologist. 2011. 16(9):1316-24. [Medline]